Multiple system atrophy variant with severe hippocampal pathology
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- Takashi Ando
- Department of Neurology Nagoya University Graduate School of Medicine Nagoya Japan
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- Yuichi Riku
- Department of Neurology Nagoya University Graduate School of Medicine Nagoya Japan
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- Akio Akagi
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Hiroaki Miyahara
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Mitsuaki Hirano
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Toshimasa Ikeda
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Hiroyuki Yabata
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Ryuichi Koizumi
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Chisato Oba
- Department of Neurology Nagoya Daini Red Cross Hospital Nagoya Japan
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- Saori Morozumi
- Department of Neurology Nagoya Daini Red Cross Hospital Nagoya Japan
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- Keizo Yasui
- Department of Neurology Nagoya Daini Red Cross Hospital Nagoya Japan
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- Atsuko Goto
- Department of Neurology National Hospital Organization Higashinagoya National Hospital Nagoya Japan
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- Taiji Katayama
- Department of Neurology National Hospital Organization Higashinagoya National Hospital Nagoya Japan
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- Satoko Sakakibara
- Department of Neurology National Hospital Organization Higashinagoya National Hospital Nagoya Japan
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- Ikuko Aiba
- Department of Neurology National Hospital Organization Higashinagoya National Hospital Nagoya Japan
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- Motoko Sakai
- Department of Neurology National Hospital Organization Suzuka National Hospital Suzuka Japan
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- Masaaki Konagaya
- Department of Neurology National Hospital Organization Suzuka National Hospital Suzuka Japan
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- Keiko Mori
- Department of Neurology Oyamada Memorial Spa Hospital Yokkaichi Japan
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- Yasuhiro Ito
- Department of Neurology Toyota Memorial Hospital Toyota Japan
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- Hiroyuki Yuasa
- Department of Neurology Tosei General Hospital Seto Japan
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- Masayo Nomura
- Department of Neurology Kainan Hospital Aichi Prefectural Welfare Federation of Agricultural Cooperatives Yatomi Japan
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- Kristine Joyce L. Porto
- Department of Molecular Neurology Graduate School of Medicine The University of Tokyo Tokyo Japan
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- Jun Mitsui
- Department of Molecular Neurology Graduate School of Medicine The University of Tokyo Tokyo Japan
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- Shoji Tsuji
- Department of Molecular Neurology Graduate School of Medicine The University of Tokyo Tokyo Japan
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- Maya Mimuro
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Yoshio Hashizume
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Masahisa Katsuno
- Department of Neurology Nagoya University Graduate School of Medicine Nagoya Japan
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- Yasushi Iwasaki
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
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- Mari Yoshida
- Department of Neuropathology Institute for Medical Science of Aging Aichi Medical University Nagakute Japan
抄録
<jats:title>Abstract</jats:title><jats:p>The striatonigral and olivopontocerebellar systems are known to be vulnerable in multiple system atrophy (MSA), showing neuronal loss, astrogliosis, and alpha‐synuclein‐immunoreactive inclusions. MSA patients who displayed abundant neuronal cytoplasmic inclusions (NCIs) in the regions other than the striatonigral or olivopontocerebellar system have occasionally been diagnosed with variants of MSA. In this study, we report clinical and pathologic findings of MSA patients characterized by prominent pathologic involvement of the hippocampus. We assessed 146 consecutively autopsied MSA patients. Semi‐quantitative analysis of anti‐alpha‐synuclein immunohistochemistry revealed that 12 of 146 patients (8.2%) had severe NCIs in two or more of the following areas: the hippocampal granule cells, cornu ammonis areas, parahippocampal gyrus, and amygdala. In contrast, the remaining 134 patients did not show severe NCIs in any of these regions. Patients with severe hippocampal involvement showed a higher representation of women (nine women/three men; Fisher's exact test, <jats:italic>p</jats:italic> = 0.0324), longer disease duration (13.1 ± 5.9 years; Mann–Whitney U‐test, <jats:italic>p</jats:italic> = 0.000157), higher prevalence of cognitive impairment (four patients; Fisher's exact test, <jats:italic>p</jats:italic> = 0.0222), and lower brain weight (1070.3 ± 168.6 g; Mann–Whitney U‐test, <jats:italic>p</jats:italic> = 0.00911) than other patients. The hippocampal granule cells and cornu ammonis area 1/subiculum almost always showed severe NCIs. The NCIs appeared to be ring‐shaped or neurofibrillary tangle‐like, fibrous configurations. Three of 12 patients also had dense, round‐shaped NCIs that were morphologically similar to pick bodies. The patients with Pick body‐like inclusions showed more severe atrophy of the medial temporal lobes and broader spreading of NCIs than those without. Immunohistochemistry for hyperphosphorylated tau and phosphorylated TDP‐43 revealed minimal aggregations in the hippocampus of the hippocampal MSA patients. Our observations suggest a pathological variant of MSA that is characterized by severe involvement of hippocampal neurons. This phenotype may reinforce the importance of neuronal alpha‐synucleinopathy in the pathogenesis of MSA.</jats:p>
収録刊行物
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- Brain Pathology
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Brain Pathology 32 (1), 2021-07-13
Wiley
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詳細情報 詳細情報について
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- CRID
- 1360857593683073408
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- ISSN
- 17503639
- 10156305
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- データソース種別
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